Waldenström's macroglobulinemia medical therapy

Risk stratification determines the protocol of management used for Waldenstrom’s macroglobulinemia. There is no treatment for asymptomatic Waldenstrom’s macroglobulinemia. The mainstay of treatment for symptomatic Waldenstrom’s macroglobulinemia is Rituximab +/- Chemotherapy. Hyperviscosity syndrome is a medical emergency and requires prompt treatment with plasmapheresis. Drug of choice for the treatment of bing-neel syndrome is Ibrutinib with or without concurrent rituximab. Other treatment options include targeted therapy, immunotherapy and radiation therapy.

There’s no cure for WM with current therapies. Instead, the treatment goals are to control symptoms and prevent end-organ damage, while maximizing quality of life. There is no standard therapy for the treatment of WM. While various drugs and combinations have demonstrated to have provided clinical benefit, hence, there are several different options for treating Waldenstrom’s macroglobulinemia depending on stage of the disease:^[1]

There is no treatment for asymptomatic patients with WM. As WM develops slowly and may not need to be treated right away, it is monitored by healthcare team every 3-6 months which is known as watchful waiting/active surveillance and treatment is started when symptoms appear, such as hyperviscosity syndrome, or there are signs that the disease is progressing more quickly.^[2] Active surveillance includes monitoring of the following laboratory parameters:

• Complete blood count (CBC) with differential
• Complete metabolic panel (CMP)
• Immunoglobulin levels in the serum (quantitative)
• Serum protein electrophoresis

Symptomatic patients with WM are started on chemotherapy depending on the stage.^[3]

• Initial stage of WM is associated with:

• Neuropathy
• Anemia or cytopenias
• Low-volume nodal involvement
• Asymptomatic splenomegaly

• Late stage of WM is associated with:

• Adenopathy
• Symptomatic splenomegaly
• Cytopenias
• Hyperviscosity syndrome
• Neuropathy
• Constitutional symptoms

• Men and women with childbearing potential should receive counseling about the potential effect of treatment on their fertility and options for fertility–preserving measures.

• Chemotherapy drugs that may be used with or without prednisone include:
  • Chlorambucil (Leukeran)
  • Fludarabine (Fludara)
  • Bendamustine (Treanda)
  • Cyclophosphamide (Cytoxan, Procytox)

• Combinations of chemotherapy drugs that may be used include:
  • DRC – dexamethasone (Decadron, Dexasone), rituximab (Rituxan) and cyclophosphamide
  • BRD – bortezomib (Velcade) and rituximab, with or without dexamethasone
  • CVP – cyclophosphamide, vincristine (Oncovin) and prednisone
  • R-CVP – CVP with rituximab
  • Thalidomide (Thalomid) and rituximab

• Waldenstrom’s macroglobulinemia complicated with hyperviscosity syndrome is a medical emergency and requires prompt treatment with plasmapheresis.^[3]
• Plasmapheresis temporarily lowers IgM levels by removing some of the abnormal IgM from the blood, which makes blood thinner.
• Plasmapheresis is usually given until chemotherapy starts to work.
• Plasmapheresis is combined with chemotherapy to control the disease for a longer period of time.
• Plasmapheresis is also used in WM patients with hemolysis.

• Many recent studies have shown to be Ibrutinib (560mg), an oral Bruton’s tyrosine kinase inhibitor, with or without concurrent Rituximab, as a drug of choice for the treatment of Bing-Neel syndrome. It works by penetrating the blood brain barrier.^{[4][5][6][7][8]}

• One or more of the following treatments can be given for Waldenstrom’s macroglobulinemia.

• Targeted therapy uses drugs to target specific molecules (such as proteins) on the surface of cancer cells. These molecules help send signals that tell cells to grow or divide. By targeting these molecules, the drugs stop the growth and spread of cancer cells while limiting harm to normal cells.
• Targeted therapy drugs used alone or in combination to treat Waldenstrom’s macroglobulinemia include rituximab, bortezomib and ibrutinib (Imbruvica).

• Immunotherapy works by stimulating, boosting, restoring or acting like the body’s immune system to create a response against cancer cells. Immunomodulatory drugs are a type of immunotherapy that interferes with the growth and division of cancer cells.
• Thalidomide is a type of immunomodulatory drug that may be used to treat Waldenstrom’s macroglobulinemia.

In some rare cases, external beam radiation therapy may be required to treat WM that develops outside of the lymphatic system (called extralymphatic disease).

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